Emergency department care of patients with Duchenne muscular dystrophy

Patients with Duchenne muscular dystrophy are living longer and are increasingly seen in Emergency Depart-ments. Though the most common cause of death remains progressive respiratory failure, increased life expectan-cies have unmasked the significance of progressive myocardial dysfunction, now associated with nearly 40% of mortalities in the DMD population. Cardiac complications such as arrhythmias and cardiomyopathy are becom-ing ever more widely recognized. Emergency physicians may encounter DMD patients with untreated, undiag-nosed or worsening of known heart disease. This review will initially familiarize the emergency physician with the pathophysiology and lifetime trajectory of care for these patients before describing specific emergency de-partment evaluation and treatment.(c) 2022 Published by Elsevier Inc.

Automated summary

Patients with Duchenne muscular dystrophy are living longer, leading to an increased presence in emergency departments. While respiratory failure remains the most common cause of death, extended life expectancies have highlighted the significance of progressive myocardial dysfunction, which is now associated with nearly 40% of mortalities in the DMD population. Cardiac complications such as arrhythmias and cardiomyopathy are also becoming more recognized. Emergency physicians may encounter DMD patients who have not been treated, diagnosed, or have worsening heart disease. This review aims to familiarize emergency physicians with the pathophysiology and lifetime care trajectory of these patients before discussing specific emergency department evaluation and treatment.